Case 1: Progressive Hypotonia and Decreased Alertness in an 8-month-old Girl

Megan Paul; Kareem Shehab; Tien Nguyen

doi:10.1542/pir.2014-0137

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Megan Paul, MD*
Kareem Shehab, MD*
Tien Nguyen, MD*

^*Department of Pediatrics, University of Arizona, Tucson, AZ

AUTHOR DISCLOSURE
Drs Paul, Shehab, and Nguyen have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device. Dr Nguyen is now in private practice at Desert Pediatrics in Tucson, Arizona and Dr Paul is a Pediatric Hematology-Oncology fellow at University of California, San Diego.

Presentation

A previously healthy 8-month-old girl in southern Arizona is admitted for progressive hypotonia and decreased alertness over several weeks. She has experienced 3 months of cough and 1 week of fever. She no longer crawls, makes eye contact, visually tracks, or babbles. Her mother travels frequently with the child to Mexico and has a chronic cough for which she has neither sought nor received care.

Her vital signs are as follows: temperature, 98.6°F (37°C); blood pressure, 101/57 mm Hg; heart rate, 117 beats/min; respiratory rate, 26 breaths/min; and oxygen saturation, 100% on room air. Her weight is in the 27th percentile for age, length is in the 90th percentile, and head circumference is in the 88th percentile. Physical examination reveals an alert but hypotonic infant, unable to support herself sitting, and with limited spontaneous movements. Sucking and swallowing are weak, gag reflex is intact, and deep tendon reflexes are 1+. Her anterior fontanelle is open, flat, and wide. Pupils are equal and reactive, but there is no visual fixation. Extraocular movements are intact. Fundi are normal. Examination of the lungs reveals …